Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Isleans Mrcog Part 2 > Thalassaemia > Flashcards

Thalassaemia Flashcards

1
Q

Define thalassaemia major

A

Homozygous for beta thalassaemia

2x defective beta globulin genes

Severe iron deficiency anaemia requiring >7 transfusions a year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Define beta thalassaemia intermedia

A

Homozygous for beta thalassaemia
2x defective beta globulin genes

Spectrum with variable transfusion requirements - <7 a year

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Describe the spectrum of disease severity seen with thalassaemia intermedia

A

Severe: diagnosed 2-6yr, with impaired growth and development

Mild: diagnosed in adulthood, often incidental finding with hepatosplenomegaly and mild anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Define thalassaemia minor

A

Heterozygous defective beta globulin gene

Mild to moderate microcytic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the effect of iron overload on the anterior pituitary

A

Delayed or incomplete puberty

Hypogonadotrophic hypogondadism
Leading to anovulatory infertility

Low bone mass due to hypo-oestrogenic state

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the cause of death in 50% of people with thalassaemia major

A

Cardiac due to iron overload

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Regarding thalassaemia major

What contraception is contraindicated

A

None

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Regarding thalassaemia major

What chelating agents can be used in pregnancy

A

None in first trimester

Desferrioxamine in second and third trimester

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Regarding thalassaemia major

What monitoring is used for diabetes

What preconception target is used

A

Fructosamine

Aim for <300 for 3/12 prior conception

Equivalent to hba1c of 43

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Regarding thalassaemia major

What are the important cut off values when assessing cardiac iron

A

Aim for T2 cardiac MRI >20ms

If <10ms high risk of cardiac failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Regarding thalassaemia major

Which patients are particularly at risk of arrhythmias

A

Older, previous significant iron overload since cleared

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Regarding thalassaemia major

What are the important cut off values in assessing liver iron overload

A

Aim for liver iron <7mg/g

If >15mg/g increased risk of cardiac overload, therefore chelation preconception or 20-28 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Regarding thalassaemia major

What % have RBC alloimmunity

A

16%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Regarding thalassaemia major

What vaccines are required and how often

A

Hep B if transfusion requirements

Pneumococcus every 5 yr
Haemophilus influenza if not previous
Meningococcal c if not previous

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Regarding thalassaemia major

If diabetic how often should fructosamine be tested

A

Monthly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Regarding thalassaemia major

How often should patients be reviewed antenatally

A

Monthly till 28 weeks then every two weeks till delivery

17
Q

Regarding thalassaemia major

When should they have US scans in the antenatal period

A

7-9 weeks due to increased risk of early Pregnancy loss

Routine dating and anomaly

Growth scans 4 weekly from 24 weeks

18
Q

Regarding thalassaemia major

What pre transfusion Hb should you aim for

A

Hb 100

19
Q

Regarding thalassaemia intermedia

When should you consider starting regular transfusions

A

FGR

Worsening anaemia

20
Q

Regarding thalassaemia intermedia

If transfusing
What pre transfusion Hb should be aimed for
How many units should initially be given

A

Aim for pre transfusion Hb of 100

Start with 2-3 units

21
Q

Regarding thalassaemia intermedia

If transfusing

How often should Hb be monitored
How many units should be given if Hb <100

A

Every 2-3 weeks

2 units of Hb <100

22
Q

Regarding thalassaemia intermedia

If not transfusing regularly what Hb cut off should be used to transfuse

A

If Hb <80 weeks give 2 units at 37-38 weeks

23
Q

Regarding thalassaemia

What thromboprophylaxis should be given

A

If splenectomy or platelets >300 give aspirin

If splenectomy & platelets >300 give aspirin & LMWH

24
Q

Regarding thalassaemia

Which patients are at highest risk of thrombosis

A

Splenectomy and no regular transfusions

High levels of peripheral RBC fragments

25
Q

Regarding thalassaemia patients

What is the intrapartum management

A

Continuous CTG monitoring

If Hb <100 cross match 2 units

If transfusion dependant desferrioxamine throughout labour - 2g over 24 hours

Active management third stage

26
Q

Regarding thalassaemia patients

What thromboprophylaxis should be given postpartum

A

LMWH
7 days following d/c if NVD

6 weeks following d/c if LSCS

27
Q

Regarding thalassaemia patients

When should desferrioxamine be restarted

A

As soon as 24hr IV infusion completed if breastfeeding

If not breastfeeding continue IV until discharge, then resume pre pregnancy iron chelation therapy

28
Q

What is the most accurate screening blood indices for thalassaemia trait

A

MCH mean corpuscular haemoglobin

Isleans Mrcog Part 2 (26 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions